CF and RhDNase

Question

Hello, I have previously read the question and the response on this topic (November 2010, Pr JC Dubus). I am the grandfather of a 6 years and 10 months old CF girl followed-up in a CF centre. Whereas everything was OK and the lungs were clean on the chest X-ray, she started a RhDNase treatment on the 14th November 2011 at the age of 5.5 years which was stopped 15 days later because of an important cough exacerbation (up to 150 to 200 cough episodes per hour) responsible for a fatigue and headaches. A few time later, the X-ray did not show clean lungs, whereas before the chest X-rays were normal, and a hospitalization was necessary to treat her with intravenous antibiotics during 15 days. In the summer, the chest X-ray was anew abnormal and a second hospitalization was planned in September. Finally, this hospitalization was unnecessary, a treatment with azithromycine (3 days per week) have shown good results. And now everything is OK.
I have 2 questions : 1°) Do we know other cases of young children requiring a rapid stop of RhDNase because of such adverse events? 2°) The medical staff wants to prescribe anew the RhDNase, but because of the side effects (cf the cited response of 2010) and the consecutive pulmonary consequences described above (but were they linked to RhDNase?), the parents don’t want to do that because they are afraid to experience again the previous catastrophic scenario for their child. Because of some progresses since the Pr Dubus’s response 2.5 years ago, what do you think about that? Thanks.

Answer

Hello and thanks for your question.
The aim of the RhDNase is to decrease the mucus viscosity. This treatment is usually systematically proposed to the CF children aged of 5 years in France, but also when some bacteria are present regularly in the sputum or when some radiological thoracic abnormalities are noted. The efficacy of the treatment is classically evaluated after 3 months of daily use to decide to continue or not RhDNase. Unfortunately this drug may have some side effects such as cough or bronchospasm and can be stopped prematurely as in the case of your grand-daughter. The scenario that you have described is not linked to the RhDNase use. This is the CF evolution with recurrent bronchial infection episodes and persistent bronchial inflammation which both may also induce important cough excesses. The current medical status of your grand-daughter incites your medical staff to propose anew a treatment for improving the mucus quality. Whatever is the chosen treatment (RhDNase, hypertonic saline, mannitol), a cough is possible. Taking bronchodilators before the inhalation of the treatment or using certain devices which decrease the aerosol rate may often improve the tolerance.
I hope that I have answered your questions. With my best regards,
Prof Jean-Christophe Dubus 

16.04.2013

Please find the previous answer cited in the question under:
ecorn-cf.eu/index.php?id=65&tx_expertadvice_pi1[showitem]=1042&tx_expertadvice_pi1[search]=Pulmozyme

D. d'Alquen